• Carney Triad

    Carney triad is a rare condition that describes the occurrence of three kinds of endocrine tumors in the same patient.

    The tumors comprising the triad are tumors in the gastrointestinal tract (known as gastrointestinal stromal tumors, or GIST), pulmonary chondromas, and paragangliomas. These masses grow chiefly in the stomach, the lungs, or the neuroendocrine tissues of the head, neck, and torso. Carney triad is extremely rare and is most common in females.

    Carney Triad Treatment at Dana-Farber/Boston Children's

    Children with Carney triad are treated at Dana-Farber/Boston Children's through our Endocrine-Oncology Program. Advanced cancers may also be treated through our Solid Tumor Center. Our integrated pediatric oncology service offers—in one specialized program—the combined expertise of a leading cancer center and a premier children’s hospital. We build a team to treat your child consisting of oncologists, endocrinologists, genetic counselors, and surgeons.

    Continue reading for more information about Carney triad or visit the Endocrine-Oncology Program page to learn more about our expertise or meet our treatment team.

    What are the causes and symptoms of Carney triad?

    As a parent, you undoubtedly want to know what may have caused your child’s condition. Carney triad is likely related to genetic factors, but the exact cause is unknown.

    The symptoms of Carney triad may vary from child to child and depend on where tumors are located and what kind they are. Symptoms might mimic other, more common ailments. Some symptoms may include:

    • Gastrointestinal bleeding
    • Stomach pain
    • Abdominal mass that can be felt on examination
    • Anemia (low red blood cell count)
    • Headaches
    • High blood pressure

    Because many of these symptoms can also point to other conditions, it’s important to have your child evaluated by a qualified medical professional right away.

    How is Carney triad diagnosed?

    The first step in treating your child is forming an accurate and complete diagnosis. Your child’s physician may order a number of different tests including:

    • A physical exam and complete medical history.
    • Blood and urine tests.
    • Endoscopy, a minor procedure in which a flexible camera is inserted through the mouth and into the stomach.
    • A biopsy, a tissue sample taken from the tumor. The tumor's appearance under a microscope helps doctors to make a diagnosis so the appropriate treatments can be recommended.
    • Magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the area where the tumor is located. An MRI uses a combination of large magnets, radiofrequencies, and a computer to analyze organs and structures within the body.
    • A computerized tomography scan (CT/CAT scan), an imaging technique that provides more detailed pictures than X-rays.
    • Molecular testing to determine whether the tumor is linked to specific genes.

    There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then, we will meet with you and your family to discuss the results and outline the best possible treatment options.

    What are the treatments for Carney triad?

    Treatment for your child's Carney triad will depend on a number of factors, including the type of tumors your child has and where they are located. Your child's doctor may recommend:

    • Surgery, involving biopsy and removal of the entire tumor and nearby tissue.
    • Radiation, the use of high-energy rays from a specialized machine to damage or kill cancer cells and shrink tumors. This is often used together with surgery, either before or after removal of the tumor.
    • Chemotherapy, a drug treatment that aims to destroy or shrink cancer cells, may be given before or after surgery.
      • Different groups of chemotherapy drugs work in different ways. Your child may receive chemotherapy orally, as a pill to swallow; intramuscularly, as an injection into the muscle or fat tissue; intravenously, as a direct injection into the bloodstream or IV; or intrathecally, as a direct injection into the spinal column through a needle. Often, a combination of chemotherapy drugs is used.
      • While chemotherapy can be quite effective in treating certain cancers, the drugs cannot differentiate normal healthy cells from cancer cells. As a result, there can be adverse side effects during treatment. Being able to anticipate these side effects can help the care team, child, and family prepare and, in some cases, prevent these complications from occurring, if at all possible.

    What is the latest research on Carney triad?

    Children who are treated through our Endocrine-Oncology Program benefit from the work of our basic and clinical researchers, who are striving to understand the scientific causes of endocrine cancers. Their work can result in the introduction of new treatment options. We are a world leader in translational research, bringing laboratory advances to the bedside and into doctors’ offices as quickly as possible.

    Clinical Trials

    Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. For many children with rare or hard-to-treat conditions, clinical trials provide new options.

    It’s possible that your child will be eligible to participate in one of our clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG). If your child has a progressive or recurrent tumor, she may be eligible for a number of experimental therapies available through these groups or from one of our independent clinical investigators.

    What is the long-term outlook for Carney triad?

    Children treated for Carney triad should visit a survivorship clinic yearly. Through the David B. Perini Jr. Quality of Life Clinic, our cancer survivorship clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team. In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other childhood cancer survivors.

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